Abstract

The objective of this study was to investigate the clinical features, diagnosis, treatment and outcomes of subclinical pituitary adenoma apoplexy (SPAA) in 185 consecutive patients between January 1990 and May 2007. Of the 185 patients, 133 (71.89%) underwent transsphenoidal tumor decompression and the remaining 52 patients (28.11%) underwent transcranial surgery. Preoperative and postoperative endocrinological hormone concentrations were measured in all patients. Pituitary imaging was obtained by CT scans or MRI. Follow-up outcomes were obtained from the records of outpatient visits and by telephone interviews. Visual disturbance, headache and pituitary function impairment improved significantly in all patients. Prolactinoma was the most frequent type of pituitary adenoma in our series (51.89%). SPAA usually occurred in patients with large or giant adenomas (85.95%). Postoperative follow-up ranged from 1 year to 17 years with a mean of 7.4 ± 1.6 years. The tumor recurred in 23 patients, 18 of whom were treated with postoperative radiotherapy for either residual tumors (n = 8) or recurrence (n = 10). Long-term thyroid hormone replacement was necessary in eight patients and steroid hormone replacement in six patients. The incidence of SPAA is relatively high compared with that of acute pituitary apoplexy. The exact pathogenic mechanism of SPAA remains unknown. MRI is significantly better than CT scans for detection of SPAA. Transsphenoidal decompression is safe and effective. Surgical decompression should be performed as early as possible.

Monday, September 28, 2009

WESTPORT — Crystle Nelson Chase was a competitive cyclist, bicycling 50 to 90 miles a day.

Her whole life changed when she was diagnosed with Cushing's disease at age 17.

Cushing's disease is caused by a tumor in the pituitary gland. These small tumors, which are not usually cancerous, can cause the production of too much cortisol, a hormone.

Now, after eight surgeries and a ninth scheduled this week, the UMass Dartmouth student is seeking an assistance dog from NEADS (Dogs For Deaf and Disabled Americans).

Her last two surgeries were to remove a brain tumor. Both failed.

Because of unexpected side effects from the first brain surgery, Chase, 23, said, "I can't bend over. I have chronic vertigo. Bending over is a killer. Just picking up a pencil off the floor makes me feel that my head's going to pop off. I can't drive anymore, either."

Chase lives with her husband, Dan, on a farm on Old County Road, just 2.7 miles from the UMass campus. When her husband can't take her to class, she rides a special tricycle.

"He helps a lot and that's great," Crystle said of Dan, who works as an assistant engineer.

But she recalled one day when she was home alone after her last surgery. "I had to call the EMTs. I had a hemorrhage. We don't have a doorbell. They were pounding on the door and I didn't hear them."

She said she is always exhausted and her husband has a difficult time waking her. "I think the dog will help him take care of me," adding the canine can also alert her when people are at the door.

The surgery this week is not on her brain but on an adrenal gland near her kidneys that is a major source of hormones. Chase expects it will take a couple of months to get back on her feet. Her treatment will include radiation therapy and her husband is taking time off from work to be with her.

When she returns to UMass Dartmouth after a medical leave, she will resume her studies in medical laboratory science. She wants to do Cushing's research.

Chase said the disease "normally (is) curable but, in this case, it's not," she said, adding she has a unique form.

Chase grew up in Rockport, where her mother still lives, and attended Gloucester High School.

Her husband has been helping her raise money for the NEADS dog and her mother helps with doctors' appointments.

Chase's father, who lives in Wilton, N.H., is also raising money for the assistance dog. A NEADS assistance dog costs the nonprofit organization $20,000 to raise and train. NEADS asks those who want one to raise $9,500, although it does not require it.

Chase said once she gets the assistance dog, "I'll actually be able to relax for the first time in 6½ years. My husband won't have to worry."

The dog will give her something more.

"I've always been such an independent person," she said. "Not only will I feel safer, I will be safer. It will be nice for me and it will be nice for my family to get part of our lives back."

Anyone who wishes to donate money to help Chase obtain a dog can send a check to NEADS, P.O. Box 213, West Bolyston, MA 01583. Donors should write Chase's name somewhere on the envelope and on the memo line on the enclosed check. Donations can also be made online at www.caringk9.org. Donations are tax deductible and any extra money raised will go to help others, Chase said.

Sunday, September 27, 2009

Robin, thanks for sharing this - I'm so impressed by what you wrote and by what the people on the message boards banded together and did for a fellow Cushie. A stark contrast to what someone recently said - we were "only an internet forum".

I was so amazed at how our community was able to mobilize and get something done.

I witnessed a miracle today. I can't even tell you much about it because it isn't my information to share without permission from the parties involved. I can tell you it is something that would never have happened without the loving, caring, EMPOWERED support of an online community on the Cushing's Help message boards.

You see, people got involved. Real people. People who know the illness, who know the doctors, who know where to turn for help. These folks are empowered on their own, but man, when they come together and work together, miracles are wrought. There is no way a doctor-centered site can give the information and resources the Cushing's community gives. It's simply impossible to do that for everything about which any one site tries to give information.

The body of knowledge that our support group has and shares with each other is so abundant with not one, not ten, but hundreds (maybe thousands) of caring, knowledgeable folks who read and share research, share what the good doctors have told them, and share who these good doctors are, we together know the symptoms/signs of every facet of these incredibly complex endocrine disease even better than the doctors do (with the exception of a very few).

We know the new trials, the new research, the new methods before most doctors. We know what has worked and what hasn't. We know the side effects, the bad that goes with the good in treatment, and what the options are. We know that if we need to know something, we can post it and someone will know someone who knows. In other words, there is no way one site CAN do what any of the wonderful communities like the Cushing's Help site do. Never. I can't put it all on my blog, no matter how hard I try. But if 100's of us (1000's, actually, of us) post on one site, we CAN be effective. It's the nature of what works. That's the model which works.

I hope I can tell you about this miracle someday soon. I want you to be as blessed as I am with it. This is the hope for the future of empowered e-patients--not doctor-driven, encyclopedic sites, but community-centered, real-patient sites.

Abstract Inferior petrosal sinus sampling (IPSS) of ACTH with CRH stimulation helps distinguish pituitary ACTH-dependent Cushing’s syndrome from the ectopic ACTH syndrome (EAS). The usefulness of the paradoxical response of other pituitary hormones including prolactin to CRH remains controversial. Data from 33 IPSS procedures carried out at the Walton Centre for Neurology and Neurosurgery in Liverpool were analyzed. Patients were selected for this procedure if they had been diagnosed with ACTH dependent Cushing’s syndrome and the majority had no obvious pituitary adenoma on Magnetic Resonance Imaging. Satisfactory simultaneous bilateral catheterization was accomplished in 23/33 (success rate 70%). The diagnostic sensitivity of a basal central/peripheral ACTH ratio >2.0 and >3 post-CRH was 94%. In two patients with subsequently confirmed EAS the maximal central/peripheral ACTH ratio was <2.0 on basal samples and did not change following CRH. The maximal central/peripheral prolactin ratio was noted at 5 min post-CRH, coinciding with the maximal central/peripheral ACTH ratio. The intersinus gradient (ISG) of ACTH was paralleled by a consistent ISG of prolactin and in 7 out of 9 patients (with successful bilateral IPSS and unilateral adenomas) the ISG of prolactin correctly lateralized the microadenoma whereas the ISG of ACTH correctly lateralized in 8 out of 9 patients. Neither of the patients with EAS achieved a central/peripheral prolactin ratio >2 in the basal state and >3 post-CRH. Bilateral catheterization of inferior petrosal sinuses can be successful in up to 70% of cases. Prolactin measurements do not have superior lateralizing capability compared with ACTH but may be useful in the differential diagnosis of pituitary-driven from EAS.

Thursday, September 24, 2009

When Richard Chole was a boy, he learned welding while helping his father refurbish a 1958 Jaguar. In fact, Chole's teachers thought he was so much better with tools than at his studies that they recommended he go to technical school rather than college.

Well, they were wrong about the studying part, but they were right about Chole's skill with his hands. Today, Chole is a surgeon and chairman of the Department of Otolaryngology at Washington University School of Medicine. He's also an inventor whose creations include a wristband warning system to prevent surgeries on the wrong site and a surgical device that allows less invasive surgery on pituitary tumors.

"Building is relaxing for me. I've always done things with my hands," said Chole, who describes himself as a car nut. He creates his inventions in a garage and superorganized, soundproof workroom in his Frontenac home, where he's also done much woodworking and other projects including constructing dollhouses for his five granddaughters.

"I'd like to build a house some day. I can do all the framing and electrical work," said Chole.

He's come a long way from grade school where he received C's and D's, the result of what he now suspects was a mild form of dyslexia. But Chole began to excel in math and science in junior high. Like his father, the first cardiologist in California's San Fernando Valley, he went on to medical school. He was recruited by Washington University in 1998 from the University of California, Davis, where he was chair of the Department of Otolaryngology-Head and Neck Surgery. When he and his family moved to St. Louis, he said his wife wanted a brick house and he wanted room for his projects and inventions. They both got their wishes.

Among the creations to emerge from that home is an alarm system called CheckSite, which helps prevent wrong surgeries.

The system uses a patient wristband with a microchip and two sensors near each operating room door. If the operating team does not mark the proper spot to operate on a patient and fails to place a sticker on the wristband to deactivate the microchip, the sensors sound an alarm. Chole got the idea for his system after a discussion at a medical staff meeting in 2004 centered on wrong-site surgeries. Shortly afterward, Chole saw security sensors in Home Depot and developed a way to track the pre-operation procedure. He initiated the patent process in spring 2004 and launched CheckSite Medical Inc., a Town and Country company formed to develop and distribute the product.

The same year he began working on an idea for a device that would allow for less invasive surgery on pituitary tumors. He came up with a speculum that's inserted up the nose and through the sinuses. Without such a device, the surgery required entry through an incision made under the lip.

Chole said he started making the device in his garage and went through about 20 prototypes before coming up with the model that's now been used in about 120 to 140 surgeries at Barnes-Jewish Hospital and St. Louis Children's Hospital.

"Necessity is the mother of invention," Chole said. "It's a safer surgery. There's no incision, no swelling."

Chole performs the surgery with Dr. Ralph Dacey Jr., Washington University's chair of neurological surgery, and Dr. Michael Chicoine, associate professor of neurological surgery. Chole does the first part of the procedure to expose the area of the tumor and the neurosurgeon removes the tumor. Chole and Dacey are working with Florida medical device maker Anspach Cos. to obtain a patent. Anspach will produce the device and there will be a royalty agreement with the surgeons, Chole said.

So what's he going to build next?

"My next project is a grandfather clock," he said. "It gets my mind off the problems of life."

Clinical decision making for patients with suspect hypercortisolisminvolves a complex diagnostic assessment. Cushing's syndromeremains one of the most challenging endocrine pathologies. Mostclinical features overlap with those of common diseases foundin the general population, and some patients have an atypicalclinical presentation with only isolated symptoms.

Recently,several studies have suggested that the prevalence of Cushing'ssyndrome is higher than previously thought. Therefore, efficientscreening tests are needed to identify the few uncovered patientsalso among unselected high-risk ambulatory patients with disorderspotentially related to cortisol excess. The recommended diagnostictests are 24-h urinary free cortisol, 1-mg overnight dexamethasonesuppression test, and late-night salivary cortisol. Once thediagnosis of Cushing's syndrome is established, the nextstep is the measurement of plasma ACTH. Then, dynamic test andappropriate imaging procedures are the most useful noninvasiveinvestigations for the differential diagnosis.

Patients withCushing's disease are generally responsive to the CRHtest and to high-dose glucocorticoid feedback. Bilateral inferiorpetrosal sinus sampling is considered the gold standard forestablishing the origin of ACTH secretion, and it is recommendedin patients with ACTH-dependent Cushing's syndrome whoseclinical, biochemical, or radiological studies are discordantor equivocal.

The present clinical case shows that even if rare,the ectopic ACTH secretion should be considered also in thosecases where the pretest probability is low.

The management ofCushing's syndrome depends on the exact knowledge of itsvarious causes, paying attention to the many potential diagnosticpitfalls. The choice of test, the modality of specimen collection(blood, urine, and saliva), the quality of measurement (assaymethodology and standardization), and close dialogue among endocrinologists,chemical pathologists, and neuroradiologists are key factorsfor optimal care of patients.

Friday, September 18, 2009

Way back, when I knew nothing about Cushing's and getting support from other who suffered as I did, I started a site...and another and another.

I suffered alone with my Cushing's for many years.

Now I have kidney cancer and I'm 3 years post-op. I have never talked with another person who has this and I'd like to. I go to a lot of classes at my local cancer support center but most people are dealing with other types of cancer and they've said that mine is too rare to have a specific group.

So... I started a "meet-up" group for kidney cancer survivors. I don't know if anyone else will be interested but I have to try. I know that the "average' kidney cancer patient is older than I am, a black male with polycystic kidneys.

There must be other of us. There are always people in the waiting room of my surgeon's office.

Please come join me and let me know that I'm not alone with this cancer, too. Maybe together we can help ourselves and not be so alone with the suffering, the waiting, the scans and surgeries.

This is a list that's going around Facebook. I know if I post it here, my non-FB friends as well as those on FB will read it and maybe add it to their blogs, as well.

1. The illness I live with is: I have 2. Post-op Cushing's Disease gives me panhypopituitarism and adrenal insufficiency. I also have post-op kidney cancer (renal cell carcinoma).

2. I was diagnosed with it in the year: Cushing's, 1985; cancer 2006

3. But I had symptoms since: About 1982

4. The biggest adjustment I’ve had to make is: Cushing's - spending most of my time sleeping; cancer - not being able to take my arthritis meds.

5. Most people assume: that I'm fine but lazy.

6. The hardest part about mornings are: waking up and moving any joints.

7. My favorite medical TV show is: House.

8. A gadget I couldn’t live without is: used to be my computer. Now my iPhone.

9. The hardest part about nights are: bad dreams.

10. Each day I take __ pills & vitamins: 2. My kidney surgeon won't let me take any more without his approval because they could harm my one kidney. One of the ones I do take is to support the kidney, the other is to support the one adrenal.

11. Regarding alternative treatments I: have a long-term anger at a practitioner who I think contributed to my father's death by not telling him he had cancer rather than stringing him along.

12. If I had to choose between an invisible illness or visible I would choose: Visible.

13. Regarding working and career: I don't have the energy to work, not full-time, anyway. I had a part-time job last year and it really sapped my non-existant energy.

14. People would be surprised to know: that I have trouble dealing with so many things.

15. The hardest thing to accept about my new reality has been: that I can't do what I want to do.

16. Something I never thought I could do with my illness that I did was: that part-time job.

17. The commercials about my illness: do not even exist.

18. Something I really miss doing since I was diagnosed is: I miss being SuperWoman. We used to have "project days", get things done. Now, I can sometimes start something but never finish.

19. It was really hard to have to give up: my arthritis meds. When I have a headache, my headache meds. Sometimes I wonder if protecting the kidney is worth the other pain.

20. A new hobby I have taken up since my diagnosis is: Naps. Sleeping. Resting.

21. If I could have one day of feeling normal again I would: Wow, I don't even know. Maybe just go shopping at the mall. I haven't done that for nearly 30 years. Maybe go work out at a gym, ride a bike. Maybe...?

26. When someone is diagnosed I’d like to tell them: Learn everything you can. Get the best doctors. This is your life, you have to take excellent care of it.

27. Something that has surprised me about living with an illness is: that I could start/run/maintain the Cushing's family of sites.

28. The nicest thing someone did for me when I wasn’t feeling well was: trying to understand.

29. I’m involved with Invisible Illness Week because: I'd like healthy people to know that there are lots of others out there who have invisible illnesses and don't make assumptions that they're just lazy, stupid, ugly, whatever.

30. The fact that you read this list makes me feel: like you care. Thank you!

Find out more about National Invisible Chronic Illness Awareness Week and the 5-day free virtual conference with 20 speakers Sept 14-18, 2009 at www.invisibleillness.com

I've been killing myself the the last 8+ years, working for an average of 4 hours or so a day AND paying for most everything on the Cushing's sites. Some donations come in but not nearly enough to cover expenses. Don't even mention time.

I'm stuck on the non-profit paperwork. I just can't seem to get the expense spreadsheet in order. Where I pay for so much, I never really kept separate records. And guessing future expenses? Who knows what those will be. But I need to do estimates on the spreadsheet.

If I were to post this on the message boards, people would say that sure, they'd help. But then they'd disappear. I've seen it time and time again.

I understand that they have lives, they're sick, other things to do.

Why am I so obsessed with doing things that nobody else even cares about? If I stop - and I'm seriously thinking of it - I don't think anyone would notice that I wasn't doing, being, providing anymore.

Even on vacation, DH was working half days, so I was, too. Bios, answering emails, board moderating, news items for the newsletter and Cushie.info, this blog and the other blogs I do.

If I stop, I don't know what I'd do with my days, though - maybe finally get the house clean?

I was just going to say I'd take the rest of this week off as a trial, then I remembered - there's an interview tomorrow night, then formatting that for iTunes.

To investigate the influence of preoperatively elevated growth hormone to bone mass in acromegalic patients, bone mineral density (BMD), and urinary type I collagen N-telopeptide were analyzed in postoperative patients with somatotroph adenomas (SA), in comparison with patients with clinically nonfunctioning adenomas (NF). The mean T and z scores of BMD in the radius of postoperative patients with SA were significantly higher than those of postoperative patients with NF. There was no significant difference in the mean T and z scores of BMD in the lumbar spine between postoperative patients with SA and those with NF. Thus, BMD in the radius is preferentially maintained in postoperative patients with SA. This result suggests that the preoperative elevation of growth hormone and insulin-like growth factor-1 are beneficial in the maintenance of bone mass in acromegalic patients.

from http://journals.lww.com/theendocrinologist/Abstract/2009/09000/Preoperative_Elevated_Serum_Growth_Hormone_is.3.aspx

Pituitary Awareness Week takes place during the week of 20-26 September 2009. Lynda Lloyd, a volunteer for the Pituitary Foundation, has succeeded in securing a place on the plinth in Trafalgar Square to mark Pituitary Awareness Week. The plinth, the empty "Fourth Plinth" in Trafalgar Square, is currently a "living art monument" that will continue 24 hours a day for 100 days. Lynda will be on the plinth, wearing a Pituitary Foundation t-shirt, on Saturday, 26 September 2009 from 11:00 – 12:00. If you are in London or the surrounding area, please go to Trafalgar Square and give Lynda some support. For those of you not close to London, you can watch Lynda on the plinth live on the web at the link below.

There are plenty of other activities going on during Pituitary Awareness Week. For full details, visit the Pituitary Foundation website below.

It’s our 15th Anniversary this year and we have decided to mark this milestone in a big way by letting people know who we are, what we do and what issues matter to our community. Our campaign this year is called:

15 Years - 15,000 Letters - £15,000

and it consists of two stages. Stage One is to send out Campaign Letters and Stage Two is aimed at raising funds.

Stage One – Campaign Letters

We need the pituitary community to band together and raise their voices (or lift their pens) to let the relevant people know the issues we face and what they can do to help us - so we’re asking you (and your partner, your family, your friends and your colleagues) to send campaign letters during Awareness Week to people who can make an immediate difference.

We have produced seven Campaign Letters to inform different people about important issues:

All we are asking is for you to personalise the letters and send them to your GP, your GP practice nurse, your local PCT, your local A&E, your local ambulance service, your Endocrinologist, and your endocrine nurses.

Our aim is to send 15,000 letters and, once Awareness Week starts, we will be asking you all to let us know how many letters you have sent and we will publish a running total on our web site.

You will not be able to download the Campaign Letters until closer to the start of Awareness Week but you can download the Campaign Letters Guidelines now – this will enable you to plan your campaign and ensure that you mark the important dates on your calendar!

The seven Awareness Week Campaign Letters are now available for you to download. Please remember, do not send out any letters until the start of Awareness Week (20th September). Please send as many letters as you can and let us make this year's Awareness Week something special. Thank You.

Following the success last year of the Strawberry Line Walk (the walk between Yatton and Cheddar) we are organising it again this year but we really want to encourage you to organise local events to raise money for The Foundation – our target is £15,000.

Cardiff - a sponsored walk from Penarth to Cosmeston Park Lakes on Saturday September 26th. Walk between half to 3.5 miles. (Click here for more details)

We need you to do something in your local area

We aren’t asking for your walk to be 25 miles with 200 people participating – you can walk as far as you want with as many (or few) people as can do it with you. Last year, the Salisbury Group organised a short, easy walk around a local landmark (Old Sarum Castle) and ten walkers raised £2000 between them – if every group could do something like this, we would easily exceed our £15,000 target! Just get sponsorship to do it (and see if your employer will match it) and let your local paper know what you’re doing and why.

Not into walking?

Then swim, bike, climb, have a coffee morning, hold a quiz, ask friends to dinner but make them pay a tenner – do anything to raise cash and get the word ‘pituitary’ out and about as far as you can.

Be creative!

Not into asking for money? Then do something like what the Sussex County Local Pituitary Support Group is doing. They’ve booked awareness days (five of them!) at local shopping centres where they’ll be displaying leaflets and information and having a draw to ‘Name the Lion’ (winner gets the soft toy lion that’s been named).

Please let us know what you’re doing so we can inform others how they can support you and we can monitor the impact our community is making!

Donations

We have created a dedicated page on the JustGiving website where you can make donations to the Awareness Week campaign (please enter on the Comment line the person/event you are sponsoring). The 'money thermometer' on the page will let eveyone keep track of the money raised so far. Simply click on the £ button to go to the JustGiving web page:

Don’t ask what The Foundation can do for you… …do something for yourself and get involved!

Together, we can make a statement, far and wide, loud and clear, to help you along your journey but also to help those who will be diagnosed in the years to come to have a better experience.

Patients with severe GH deficiency (GHD) suffer with a reduced quality of life in addition to diverse changes in cardiac size and performance. So far, the cardiac reserve ability to maintain the circulation during peak exercise has not been measured. We tested the hypothesis that patients with severe GHD have reduced cardiac reserve function compared with healthy controls and that this could explain, in part, their reduced quality of life. Aims

Using this robust noninvasive method of assessing functional cardiac pumping capacity, we have for the first time shown that, while patients with severe GHD have a significantly impaired cardiac functional reserve associated with chronotropic incompetence and impaired pressure-generating capacity, this does not correlate with their reduced quality of life assessed using the current standard AGHDA score.

Document Type: Research article

DOI: 10.1111/j.1365-2265.2009.03560.x

Affiliations:1: Department of Endocrinology, Leeds General Infirmary 2: Academic Unit of Molecular Cardiovascular Research, University of Leeds and Yorkshire Heart Centre, Leeds General Infirmary, Leeds 3: Human Development and Ageing Research Unit, School of Sport and Exercise Science, John Moore's University, Liverpool, UK

The full text electronic article is available for purchase. You will be able to download the full text electronic article after payment.

By JOSEPH BROWNSTEIN ABC News Medical Unit

A survivor of brain surgery, Kellie Graham considers herself lucky because she was diagnosed early, because she received advanced medical care and because she had a type of tumor that could be removed through her nose.

Graham had a tumor at the base of her brain, a pituitary tumor that was removed via a relatively novel technique known as endoscopic brain surgery. In this procedure, an endoscope is put through the nasal passages and an incision is made in the back by an otolaryngologist -- an ear, nose and throat surgeon -- and then handed off to a neurosurgeon to remove tumors at the base of the brain.

"I felt really comfortable with it because both physicians explained to me exactly what they were going to do," said Graham, describing how she felt after she learned about the surgery.

She also was aided, she said, by the reassurances of a friend who had undergone a similar operation before.

"That was probably why I was not as apprehensive as I could have been," said Graham.

While the surgery treats a serious condition, surgeons who perform the operation say it is a more optimal treatment than some of those previously used.

"You're going through the nose, you're doing some dissection, but your tumor's right there," said Dr. Ameet Singh, an otolaryngologist and co-director of endoscopic pituitary and skull base surgery at the George Washington University Hospital, who performed Graham's operation. "You save the patient not only a lot of pain, but a lot of time in terms of their recovery period."

The procedure is one of many that have progressed in recent years and offer less-invasive operations, such as kidneys that are removed through the belly button. It is performed at a number of medical centers.

"This is a technique that has grown over the past five years through a close collaboration between ENT surgeons and neurosurgeons," said Dr. Theodore Schwartz, a neurosurgeon at New York-Presbyterian Hospital and Weill Cornell Medical Center.

But while the particular operation Graham had was relatively novel, the notion of tumor removal through the nasal passages is not.

"The whole concept of removing pituitary tumors through the nose probably goes back to the 1960s -- it's probably been around 30 or 40 years," said Dr. Keith Black, director of the Cedars-Sinai Maxine Dunitz Neurosurgical Institute.

Brain tumors were initially treated by removing a piece of the skull, and in later years, procedures were done utilizing a microscope, which gave an enlarged view of the tumor and the surrounding area.

Black said that using the endoscope in place of the microscope presents a tradeoff -- advantageous in some aspects but a disadvantage in others.

With the endoscope, he said, the operation is a bit less invasive because the incision is made further back in the nasal passages.

At the same time, he said, "The disadvantage of the endoscope is that, with an endoscope, you're relying on a camera to look at the image."

In such a situation, he said, some depth perception is compromised, even with improved cameras that have higher definition.

"You don't quite have a three-dimensional image; you don't quite get three-dimensional depth," he said.

Endoscopic brain surgery is not available for all tumors in the brain, just those at the base.

Tumors like gliomas -- the type of tumor that afflicted the late Sen. Edward Kennedy -- would not be treatable with this procedure because they are located elsewhere in the brain.

Risks and Rewards

As with any procedure, endoscopic brain surgery carries its share of risks.

One risk, noted Schwartz, was that of infection, given that the surgical tools need to pass through relatively dirty nasal passages into a sterile brain area.

"You need to be very meticulous [about] how you close the skull base after surgery to make sure the opening you made is well-closed," he said.

Also for that reason, said Schwartz, antibiotics are given to the patient.

But, he said, that type of complication is uncommon.

"They don't get infections at a high rate," said Schwartz.

In her own case, Graham said she was told she needed a lumbar drain placed at the base of her spine because of the potential risk of spinal fluid draining into her skull.

"You just do something in a precautionary manner so if there is a leak, you've already taken care of it," said Graham.

Black noted that one of the advantages of the surgery could prove to be a disadvantage, since the incision is further back in the nasal passages.

"If there's a major injury, if you get major bleeding, it's harder to control with the endoscope than the microscope," said Black.

He noted, however, that doing surgeries through the nose has provided benefits overall, even if he considers the endoscope to be only an incremental advance.

Few procedures, Black said, now require cutting into the skull.

"That approach now is reserved for very large tumors that we cannot remove through the nose," he said.

At this point, he estimated, 97 percent of pituitary tumors are done through the nose, not by removing the skull.

Dr. Vijay Anand, an otolaryngologist who performs the operation with Schwartz at New York-Presbyterian Hospital, was more enthusiastic.

"It's a minimally invasive procedure with maximum benefits," he said.

Brain Surgery on the Ground

While endoscopic brain surgery may provide some advantage for people, the biggest beneficiaries of endoscopic brain surgery may walk on four legs.

Lisa Klopp, a veterinary neurosurgeon at the Sams Clinic in San Francisco, learned from a neurosurgeon, who worked on humans, in order to perform endoscopic brain surgery on cats and dogs.

"One of the difficulties with doing brain surgery in dogs and cats is the size of the cranial vault," she said.

The endoscope, she said, has allowed her to fully remove tumors that she could not have fully accessed previously because she could not fully maneuver in the skull of the animal.

"[The endoscope] allows a very good light into an area I wouldn't be able to see into," said Klopp. "Definitely, the majority of tumors I have to do, I wouldn't' be able to see into the areas with a microscope."

The Human Angle

Graham said that the greatest benefit she received in preparing for the surgery was one not available to pets.

"The more information they can have about the procedure, it just really puts them at ease," she said of patients preparing for the operation.

In her own case, Graham said, the tumor had been pressing on her optic nerve, impairing her vision, and after the surgery, the main side effect she noticed was congestion.

Overall, she said, another of the prime benefits she received was excellent care from attentive nurses, something she said is not emphasized enough when people discuss health care.

"My situation was very positive," said Graham. "I'm glad it's over and I'm glad it turned out really well."

Clinical decision making for patients with suspect hypercortisolism involves a complex diagnostic assessment.

Cushing's syndrome remains one of the most challenging endocrine pathologies. Most clinical features overlap with those of common diseases found in the general population, and some patients have an atypical clinical presentation with only isolated symptoms. Recently, several studies have suggested that the prevalence of Cushing's syndrome is higher than previously thought.

Therefore, efficient screening tests are needed to identify the few uncovered patients also among unselected high-risk ambulatory patients with disorders potentially related to cortisol excess. The recommended diagnostic tests are 24-h urinary free cortisol, 1-mg overnight dexamethasone suppression test, and late-night salivary cortisol. Once the diagnosis of Cushing's syndrome is established, the next step is the measurement of plasma ACTH. Then, dynamic test and appropriate imaging procedures are the most useful noninvasive investigations for the differential diagnosis. Patients with Cushing's disease are generally responsive to the CRH test and to high-dose glucocorticoid feedback. Bilateral inferior petrosal sinus sampling is considered the gold standard for establishing the origin of ACTH secretion, and it is recommended in patients with ACTH-dependent Cushing's syndrome whose clinical, biochemical, or radiological studies are discordant or equivocal.

The present clinical case shows that even if rare, the ectopic ACTH secretion should be considered also in those cases where the pretest probability is low. The management of Cushing's syndrome depends on the exact knowledge of its various causes, paying attention to the many potential diagnostic pitfalls. The choice of test, the modality of specimen collection (blood, urine, and saliva), the quality of measurement (assay methodology and standardization), and close dialogue among endocrinologists, chemical pathologists, and neuroradiologists are key factors for optimal care of patients.

I'm still not sure that the tick living on my neck (Holy Moley!) didn't have anything to do with my current enlarged lymph node but my PCP didn't think that it did.

I'm still a bit nervous about this - I hate reading scan reports - but my PCP said if it wasn't keeping me up at night with worry, we could do another scan in November to see if the node was still growing or not. If it is, we do a biopsy then. If not, we scan again in a few months.

So, I decided to wait. My other option was doing the biopsy now. I'll put that off for the time being, thank you!

Thinking Happy Thoughts that nothing is happening that shouldn't be...

Wednesday, September 9, 2009

FOR most people diagnosed with pituitary disorders, the condition is initially something of a mystery.

Symptoms develop over time and may go unnoticed for a number of years.

Helping to unravel the medical mysteries are specialist nurses Sondra Gorick and Kathy Powell, whose work in the Clinical Investigation Unit at NNUH is key to diagnosing and monitoring pituitary disorders.

Now Sondra and Kathy are re-launching a local support group for pituitary patients sponsored by the Pituitary Foundation, a national charity, and helped by Tony Denton, a volunteer at NNUH who is also a patient.

"The pituitary is like the conductor of the orchestra - it controls all the other glands in the body such as the thyroid, adrenal glands, ovaries and testes," Sondra explains.

"A large part of our work is to reassure patients who suffer symptoms that can be extremely distressing. For instance, acromegaly is a rare condition that affects the growth hormones, causing the patients' features to grow out of all proportion. Once the hormones are controlled, either with drugs or surgery, the appearance can return to normal.

"It's thought that Goliath had this condition - in the bible story he was a giant and David was able to sneak up on him because in acromegaly the peripheral vision is also affected."

Another condition is Cushing's Disease, which is a tumour of the pituitary which makes the adrenal glands produce too much natural steroids. This is a very distressing condition because the patients put on large amounts of weight particularly around their middles. They get very weak muscles, bruise very easily, often develop diabetes and high blood pressure and several other problems.

Most tumours on the pituitary turn out to be benign and some may not require any treatment at all. However, tests and treatments have improved a great deal in recent years and patients can now look forward to longer, healthier lives with much greater control of their symptoms."

The first meeting of the relaunched support group for pituitary patients will take place 16th September in Benjamin Gooch Hall within the Teaching Centre at NNUH, from 7 pm. It's hoped that the group will meet on a three-monthly basis.

Sondra - who was recently nominated for a "Patient Choice" award in the 2009 Norfolk and Norwich University Hospital Staff Awards - is pledging her support for the Pituitary Foundation by holding a sponsored "green hair day" at NNUH on her 50th birthday, 24 November.

If you would like to know more about the Norwich pituitary support group, call Sondra Gorick or Kathy Powell on 01603 286360 or Tony Denton on 01953 605534

Tuesday, September 8, 2009

When David Clarke, MD, removed a benign tumor from a woman's brain last month, it was, in a way, the second time he had performed the surgery. Days earlier, the Halifax, Nova Scotia-based neurosurgeon had rehearsed the complex procedure on a state-of-the-art virtual-reality simulator, using a model created from the patient's own medical information. Dr. Clarke believes the operation was the first of its kind where a brain tumor surgery was practiced beforehand on a patient-specific simulator.

Known as NeuroTouch, the computer simulator is being developed by a National Research Council of Canada team of engineers, computer programmers and neuroscientists.

"We have flight simulators," says Ryan D'Arcy, PhD, a researcher at the NRC site in Halifax, and one of the "brains" behind the project, so he wanted to see "what would a simulator for neurosurgery be like."

But Dr. D'Arcy believes this machine one-ups flight simulators. "This is effectively as though your pilot would simulate the exact flight you're about to take before you get on the airplane," Dr. D'Arcy says. "They would simulate the air conditions, what is the best route or whatever."

To prepare simulations on NeuroTouch, surgeons feed MRI scans and other patient info into the computer, which then uses previously gathered data on tumor tissue types to create a three-dimensional model of the patient's condition -- all presented eerily realistic, high-resolution graphics.

The surgeon controls the virtual operation using a feedback-giving device that mimics the look and feel of the continuous ultrasonic aspirator. (Aspirators are surgical tools that vibrate at a high-frequency, pulverizing cancerous tissue, which they then clear with suction.)

"You can feel the vibration," says Dr. Clarke, "the tissue as it's being removed. It's like the actual thing."

For now, NeuroTouch can model three of the most common types of brain cancers, according to Dr. Clarke, including primary brain tumors, pituitary tumors and meningiomas. The tumor removed from the Nova Scotian woman was a meningioma, a benign tumor that afflicts about two percent of the population.

NeuroTouch will undergo trials at 5 sites across Canada, with 50 researchers from various disciplines involved in the project.

Robert DiRaddo, PhD, of the NRC site in Montreal, and the Scientific Lead of NeuroTouch, is in talks with companies to commercialize the device, but he cannot divulge who is interested. "We would like to have an agreement in place within the next one to two years," he said in a statement.

Dr. Clarke thinks the biggest impact of the simulator will be on the patients themselves. "How did my simulated surgery go?" was the first question his patient asked, he says, after he did a dress rehearsal on NeuroTouch. "It may be prophetic," he continues. "I think in the future, people and patients will want to know, how did it go? Were there problems encountered? Were you able to get around those problems? It obviously has implications for the risk of surgery, for consent of surgery. It represents [a change in] how the surgeons will interact with patients."

Objective: An important safety issue with growth hormone replacement therapy (GHRT) in hypopituitary patients with a history of a pituitary adenoma is the risk for tumour recurrence or enlargement.

Design: Case-control study.

Subjects and Methods: We studied tumour progression rate in 121 patients with hypopituitarism on the basis of non-functional pituitary adenomas (NFPA) receiving long-term GHRT. A group of 114 NFPA patients not receiving GHRT who were matched in terms of duration of follow-up, gender, age, age at diagnosis and radiotherapy status were used as a control population. The average duration of GHRT was 10 ± 4 years (range 2-17).

Results: In patients with a known residual adenoma, 63 % had no detectable enlargement of tumour during the study. In patients who had no visible residual tumour prior to GHRT, 90 % did not suffer from recurrence. In total, the 10-year tumour progression free survival rate in patients with NFPA receiving GHRT was 74 %. In the control population not receiving GHRT the 10-year progression free survival rate was 70 %. Radiotherapy as part of the initial tumour treatment reduced the rate of tumour progression in both GHRT and non-GHRT patients to a similar extent.

Conclusions: The rate of tumour progression was similar in this large group of GHRT patients and the control population not receiving GHRT. Our results provide further support that long-term use of GH replacement in hypopituitarism may be considered safe in patients with residual pituitary adenomas.

Context: Gender-atypical behavior has been described in young girls as well as in women with congenital adrenal hyperplasia (CAH) due to a CYP21A2 deficiency.

Objective: The aim of the study was to assess health-related, psychosexual, and psychosocial parameters and correlate the results to CYP21A2 genotype.

Design and Participants: Sixty-two Swedish women with CAH and age-matched controls completed a 120-item questionnaire and a validated quality of life instrument [psychological general well-being (PGWB) formula] to identify psychosexual and psychosocial parameters. The patients were divided into four CYP21A2 genotype groups.

Results: The women with CAH held more male-dominant occupations (30%) compared to controls (13%) (P = 0.04), especially those in the null genotype group (55%) (P = 0.006). They also reported a greater interest in rough sports (74%) compared to controls (50%) (P = 0.007). Eight women with CAH (14%) reported a prime interest in motor vehicles, compared to none of the controls (P = 0.002). Non-heterosexual orientation was reported by 19% of women with CAH (P = 0.005), 50% in the null genotype group (P = 0.0001), 30% in I2splice (NS), and 5% in I172N (NS). PGWB total score did not differ between patients and controls.

Conclusion: We identified increased gender-atypical behavior in women with CAH that could be correlated to the CYP21A2 genotype. This speaks in favor of dose-dependent effects of prenatal androgens on the development of higher brain functions. The impact of the disease on upbringing and interpersonal relationships did not correlate with disease severity, indicating that other factors, such as coping strategies, are important for psychosocial adaptation. This illustrates the need for psychological support to parents and patients.

Main Outcome Measures: After withdrawal of antisecretory drugs, patients were considered in remission if they had mean GH levels below 2 ng/ml and normal IGF-I (acromegaly), normal 24-h urinary free cortisol, and cortisol less than 50 nmol/liter after low-dose dexamethasone test (CD) or two consecutive normal samplings of prolactin levels (prolactinoma).

Results: After a mean follow-up of 96 months, 44.7% of the patients were in remission. Mean time to remission was 42.6 months. Twelve patients presented late remission at least 48 months after SR. Two patients with CD presented late recurrence 72 and 96 months after SR. Forty percent of patients treated primarily with SR were in remission. Target volume and initial hormone levels were significant predictive factors of remission in univariate analysis. Radiation-induced hypopituitarism was observed in 23% patients; in half of them, hypopituitarism was observed after a mean time of 48 to 96 months. Twenty-four patients were followed for more than 120 months; rates of remission and hypopituitarism were similar to the whole cohort.

Conclusions: SR is an effective and safe primary or adjunctive treatment in selected patients with secreting pituitary adenomas.

About Me

I am a Cushing's patient who has dealt with Cushing's symptoms since 1983 (or earlier) and the aftereffects of pituitary surgery since 1987.

Because I had very little support for my symptoms, diagnosis and surgery, I decided to try to make things a little better for other patients and started a support site called Cushing's Help and Support in 2000. The site has grown to astronomical numbers. This disease isn't as rare as doctors have told us!

In 2006, I was also diagnosed with kidney cancer (renal cell carcinoma). My left kidney and adrenal gland were removed. Having an adrenal gland removed complicates my post-Cushing's symptoms.